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Congenital mitral valve regurgitation in adult patients. A rare, often misdiagnosed but repairable, valve disease

^a AP-HP, Assistance Publique - Hôpitaux de Paris, Service de Chirurgie Cardiovasculaire, Hôpital Européen Georges Pompidou, Paris, France
^b Université René Descartes, Paris V, France

Received 5 November 2007; received in revised form 5 June 2008; accepted 9 June 2008.

^_* Corresponding author. Address: Hôpital Européen Georges Pompidou, Service de Chirurgie Cardiovasculaire, 20, rue Leblanc 75908, Paris, France. Tel.: +33 1 56 09 37 48; fax: +33 1 56 09 22 19. (Email: rzegdi{at}hotmail.com).

Objective: Congenital mitral valve regurgitation (MVR) is a rare disease occurring in infancy or childhood. Although congenital MVR has been described in adults, no surgical series has been reported so far. We describe here a 6-year surgical experience of congenital MVR in adults at a single institution. Methods: We reviewed the data of 15 consecutive patients (8 men), aged more than 16 years (median: 38 years; range: 16–70 years) operated on for severe congenital MVR from June 2000 to March 2006. Congenital MVR represented 2.1% of mitral valve surgery performed in adults during the same period. Patients with atrio-ventricular septal defect or atrio-ventricular discordance were excluded. Results: The congenital MVR was preoperatively diagnosed in six (40%) cases. Two (13%) patients had a Williams–Beuren syndrome. The lesions consisted in annular dilation (100%), prolapsed leaflet (87%), chordal abnormalities (80%), papillary muscle abnormalities (40%) or valvular cleft (33%). Mitral valve repair was performed in all cases using Carpentier's techniques. There was no hospital death or late mortality. At last follow-up (median: 60 months; range: 6–83 months), all patients were in NYHA functional class I or II and in a sinus rhythm. On transthoracic echocardiography, 11 (73%) patients had no or trivial MVR. Mild MVR was present in four (27%) patients. No patient was reoperated and endocarditis did not occur. Conclusion: Congenital MVR is rare in adults, often misdiagnosed and accessible to valve repair with excellent mid-term results.

Key Words: Valve • Repair • Congenital • Regurgitation